Anatomical Classification and Posttreatment Remodeling Characteristics to Guide Management and Follow-Up of Neonates and Infants With Coronary Artery Fistula: A Multicenter Study From the Coronary Artery Fistula Registry

Abstract

Background: Coronary artery fistulas (CAFs) presenting in infancy are rare, and data regarding postclosure sequelae and follow-up are limited. Methods: A retrospective review of all the neonates and infants (<1 year) was conducted from the CAF registry for CAF treatment. The CAF type (proximal or distal), size, treatment method, and follow-up angiography were reviewed to assess outcomes and coronary remodeling. Results: Forty-eight patients were included from 20 centers. Of these, 30 were proximal and 18 had distal CAF; 39 were large, 7 medium, and 2 had small CAF. The median age and weight was 0.16 years (0.01–1) and 4.2 kg (1.7–10.6). Heart failure was noted in 28 of 48 (58%) patients. Transcatheter closure was performed in 24, surgical closure in 18, and 6 were observed medically. Procedural success was 92% and 94 % for transcatheter closure and surgical closure, respectively. Follow-up data were obtained in 34 of 48 (70%) at a median of 2.9 (0.1–18) years. Angiography to assess remodeling was available in 20 of 48 (41%). I. Optimal remodeling (n=10, 7 proximal and 3 distal CAF). II. Suboptimal remodeling (n=7) included (A) symptomatic coronary thrombosis (n=2, distal CAF), (B) asymptomatic coronary thrombosis (n=3, 1 proximal and 2 distal CAF), and (C) partial thrombosis with residual cul-de-sac (n=1, proximal CAF) and vessel irregularity with stenosis (n=1, distal CAF). Finally, (III) persistent coronary artery dilation (n=4). Antiplatelets and anticoagulation were used in 31 and 7 patients post-closure, respectively. Overall, 7 of 10 (70%) with proximal CAF had optimal remodeling, but 5 of 11 (45%) with distal CAF had suboptimal remodeling. Only 1 of 7 patients with suboptimal remodeling were on anticoagulation. Conclusions: Neonates/infants with hemodynamically significant CAF can be treated by transcatheter or surgical closure with excellent procedural success. Patients with distal CAF are at higher risk for suboptimal remodeling. Postclosure anticoagulation and follow-up coronary anatomic evaluation are warranted.

Congenital Coronary Artery Fistula in Children: A Review of 28 Cases with Clinical and Imaging Outcomes

“…Furthermore, treatment strategies for thrombotic occlusion of the fistula in children are debated, unlike Kawasaki disease, which has been well-described for the management of coronary aneurysm with thrombus. The recent retrospective review of CCAF in neonate and infants [20] showed the usage of thromboprohylaxis; antiplatelets and anticoagulation in 90% of patients post closure. Suboptimal remodeling was also reported in some patients.…”

Diagnosis of left anterior descending branch‐right ventricular fistula with giant coronary artery aneurysm by contrast echocardiography: A case report

“…Because of excessive cardiac chamber load, elderly patients may develop arrhythmia and congestive heart failure 6 . Another review of CAF in neonates and infants (<1 year) showed that heart failure was noted in 28 of 48 (58%) patients 7 . Due to the abundant blood flow through the fistula, the involved coronary artery often becomes dilated, tortuous, and even develop aneurysms 8 .…”

“…6 Another review of CAF in neonates and infants (<1 year) showed that heart failure was noted in 28 of 48 (58%) patients. 7 Due to the abundant blood flow through the fistula, the involved coronary artery often becomes dilated, tortuous, and even develop aneurysms. 8 Any of the three major coronary arteries may be the feeding artery for CAFs, and the fistula varies in curvature and dilation due to the feeding artery and original point.…”

Management of a large coronary artery fistula in a neonate

“…While largely presumed to be asymptomatic, a recent study showed that a majority of neonates/infants presented with heart failure–type symptoms, albeit many of them had a larger fistula size. 2 Indications for intervention on CAFs have been controversial because many patients are asymptomatic; however, delaying intervention is associated with a significantly greater risk of death preoperatively and an increased risk of morbidity and mortality postoperatively. 3 Therefore, the majority of CAFs are addressed when they are identified.…”

“…Transcatheter and surgical approaches have been both been used with good success rates; however, the number of neonates undergoing repair by either method remains low. 2 , 4 Here, we present a case of surgical repair of a large CAF in a neonate after an attempted transcatheter closure resulted in iatrogenic tricuspid regurgitation (TR). The institutional review board of Children's Healthcare of Atlanta approved the study protocol and publication of data (approval number: STUDY00001462, date approved: July 18, 2022).…”